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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Desmoid tumor


Other Names for this Disease
  • Aggressive fibromatosis
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Overview



What is a desmoid tumor?


What is a desmoid tumor?

A desmoid tumor is the abnormal growth of cells in connective tissues, which are tissues that help to maintain the structure of the body and include the tissues covering muscle (fascia), cartilage and fat.  Though these tumors have a tendency to invade surrounding tissues and organs (be aggressive), they rarely spread to more distant parts of the body (metastasize).  Desmoid tumors occur in 2-5 people per million and are most often found in the muscles of the shoulder or abdominal wall, though they can occur in other parts of the body as well.[1]  The first signs that a desmoid tumor is growing include a growing mass or lump; if the mass presses on other parts of the body, it could cause moderate pain, numbness, tingling, or limit the movement of limbs.[2]  A desmoid tumor is usually diagnosed by magnetic resonance imaging (MRI) followed by a biopsy.  Treatment consists of surgery to remove as much of the tumor as possible; radiation therapy, chemotherapy, or hormone therapy may also be used to reduce the chance that the tumor might regrow following surgery.[1]  The cause of desmoid tumors is not completely clear;  however, approximately 5% of these tumors are associated with an inherited colon cancer syndrome known as familial adenomatous polyposis (FAP).[3]
Last updated: 10/27/2011

References
  1. Biermann JS. Desmoid Tumors. Current Treatment Options in Oncology. 2000; 1:262-266. http://www.ncbi.nlm.nih.gov/pubmed/12057169. Accessed 10/25/2011.
  2. Hosalkar HS, Fox EJ, Delaney T, Torbert JT, Ogilvie CM, Lackman RD. Desmoid Tumors and Current Status of Management. The Orthopedic Clinics of North America. 2006; 37:53-63. http://www.ncbi.nlm.nih.gov/pubmed/16311111. Accessed 10/24/2011.
  3. de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F. Desmoid tumors: need for an individualized approach. Expert Review of Anticancer Therapy. 2009; 9:525-535. http://www.ncbi.nlm.nih.gov/pubmed/19374605. Accessed 10/24/2011.